Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 53-year-old male with Cushing's syndrome due to ectopic ACTH production from medullary carcinoma of the thyroid was reported. The clinical course and results of detailed endocrinological studies and immunohistochemical findings about the cancer tissue were described. An abnormally high concentration of calcitonin, ACTH and beta-MSH in both plasma and cancer tissue (thyroid, lymph nodes and liver) were documented by radioimmunoassay. Urinary 17-OHCS was as high as 38.4 mg/day and showed no supression following dexamethasone 8 mg/day administration. ORAL METYRAPONE (3 G/DAY) CAUSED NO RESPONSE IN URINARY 17-OHCS. Parallel increments in plasma calcitonin, ACTH and beta-MSH were observed following calcium and gastrin loading. Total thyroidectomy with modified radical neck dissection caused minimal changes of plasma levels of calcitonin, ACTH and beta-MSH and no improvement in the clinical manifestations of Cushing's syndrome. An aortogram revealed metastatic tumors in the liver. A second operation, total adrenalectomy, resulted in an improvement of the clinical and laboratory findings such as hypokalemia, high blood pressure, muscle atrophy and moon face. Immunofluorescent study showed different distribution patterns in calcitonin- and ACTH-positive cells in the primary focus but similar patterns in the liver metastasis.
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PMID:[A case of medullary thyroid carcinoma with ectopic ACTH syndrome (author's transl)]. 20 14

A 29-year-old man with a marfanoid habitus, peculiar mucosal neuromas of the lips and tongue, high arched palate, hyperplastic corneal nerves, and hypertension was found at operation to have medullary carcinoma of the thyroid, parathyroid hyperplasia, and pheochromocytoma. These symptoms and findings are characteristic of multiple endocrine adenomatosis (MEA IIb) syndromes.
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PMID:Multiple endocrine adenomatosis type IIb. Diagnosis and treatment. 24 66

A 17-year-old woman manifested fever, abdominal pain, headache, and hypertension caused by a solitary, benign pheochromocytoma. She also had hypercalcemia and elevated plasma immunoreactive calcitonin levels. After removal of the pheochromocytoma, calcium and calcitonin levels returned to normal. Studies of peripheral and tumor venous blood showed no excess or ectopic parathyroid hormone secretion, but the tumor contained and secreted calcitonin. Sporadic pheochromocytoma may secrete calcitonin and cause hypercalcemia by non-parathyroid hormone-mediated mechanisms. The potential is clearly present for confusion with multiple endocrine neoplasia, type 2 (medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism).
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PMID:Pheochromocytoma associated with hypercalcemia and ectopic secretion of calcitonin. 46 64

The multiple endocrine neoplasia syndromes are an association of tumours of 2 or more endocrine glands. Multiple endocrine neoplasia type 2b (MEN 2b) patients develop medullary thyroid carcinoma and pheochromocytomas as well as unique physical characteristics. Most commonly, MEN2b is inherited with an autosomal dominant pattern although sporadic cases are not uncommon. If untreated the disease may be lethal. The facial, oral and ocular characteristics are reliable markers of the disease. These patients give a history most commonly of slipped capital femoral epiphysis, hypertension and life-long diarrhoea and/or constipation. MEN2b is most commonly characterised by nodules on the anterior aspect of the tongue, thickened lips with nodules, thickened upper eyelids, broadened nasal bridge, thickened corneal nerves and dilated, symmetrical, pedunculated nodules on the cheek mucosa. The patient described has most of these characteristics. Radiographic features of the jaws which have not been previously described are reported. These include a markedly enlarged and bifurcated inferior alveolar canal and shortened roots of the lower incisor teeth. Due to the lethality of the disease, patients who present with the above physical characteristics must be further investigated to exclude MEN2b.
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PMID:Multiple endocrine neoplasia syndrome--type 2b. Case report and review. 135 Oct 93

High plasma concentrations of neuropeptide Y (NPY) were found in a patient with bilateral adrenal phaeochromocytomas and medullary thyroid carcinoma associated with MEN IIa (32 pmol/l, normal less than 3.5 pmol/l). Both adrenal tumours contained and secreted NPY. Manipulation at operation produced a remarkable increase in plasma NPY concentrations (peak = 1631 pmol/l) coinciding with increases in plasma levels of catecholamines and arterial pressure. NPY was also shown to be present in thyroid tumour tissue: the concentration of NPY in tumour was 50-fold higher (0.9 nmol/g vs 0.004 nmol/g) than in adjacent normal thyroid tissue. It is possible that NPY from some phaeochromocytomas may contribute to hypertension during surgery.
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PMID:Neuropeptide Y in multiple endocrine neoplasia: release during surgery for phaeochromocytoma. 287 55

A 26-year-old male presented with the symptoms and signs of acute congestive heart failure and hypertension. The left ventricle was shown to be thickened and displayed reversible hypokinesia. Further investigations revealed the underlying pathology of a phaeochromocytoma, bilateral medullary thyroid carcinoma (MTC), parathyroid adenoma and macro-prolactinoma. There was a family history of MTC. The echocardiographic features of catecholamine-induced cardiomyopathy were important in this diagnosis. Our research revealed no previous report of Sipple's syndrome associated with a macroprolactinoma. This case, along with the other 13 reports of mixed (type I and II) multiple endocrine neoplasia (MEN), are not within the classical subsets of MEN.
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PMID:Sipple's syndrome associated with a large prolactinoma. 289 62

Phaeochromocytoma was diagnosed in 77 (0.36%) of 21,420 hypertensive patients examined in the hypertension units of the Broussais and Saint-Joseph hospitals, Paris, between 1976 and 1986. Our diagnostic strategy is to reserve biochemical examinations to cases with suspected phaeochromocytoma and to explore only those patients who have positive laboratory results. Patients suspected of harbouring a phaeochromocytoma are those who complain of headaches, palpitations and sweating (these 3 symptoms together having a 90.9% sensitivity and a 99.9% exclusion value), those who have a family history of phaeochromocytoma or who present with medullary thyroid carcinoma or phakomatosis, or those who do not respond to anti-hypertensive treatments. Altogether, these patients account for less than 10% of all cases of hypertension. The most sensitive test in this group is measurement of urinary metanephrines. Among 30 patients with phaeochromocytoma in whom urinary metanephrines and plasma noradrenaline were measured on the same day, none had urinary metanephrine values lower than 3.69 mumol/24 h (0.7 mg/24 h) while 6, who had normal blood pressure at the time of sampling, had noradrenaline levels below 3.53 nmol/l (600 pg/ml). Prior to surgery, the tumour was correctly located by urography (69% of 58 n = tumours), ultrasounds (74%, n = 38), arteriography (83%, n = 23), radioisotope scanning (91%, n = 32), computed tomography (95%, n = 40) and nuclear magnetic resonance imaging (12/12). In 28 patients who had both radioisotope scanning and computed tomography the sensitivities of these examinations were 90% and 100% respectively. A stage by stage approach to the diagnosis of phaeochromocytoma, using detection criteria followed by biochemistry then location methods, is an economical strategy with the best yield from diagnostic and imaging techniques.
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PMID:[Detection, diagnosis and localization of pheochromocytoma. 77 cases in a population of 21,420 hypertensive patients]. 296 16

Twenty-five patients were identified with non-pituitary, nonadrenal ACTH-secreting tumors (bronchial carcinoid, bronchial small cell carcinoma, pancreatic islet cell carcinoma, medullary thyroid carcinoma, thymic carcinoids, metastatic adenocarcinoma, and pancreatic cystadenoma). Clinical features were weakness, hypertension, cushingoid appearance, peripheral edema, personality disorders, and hyperpigmentation. Biochemical features were a markedly increased urinary free cortisol level (all patients), hypokalemia (71 percent of patients), and an elevated ACTH level (72 percent of patients). Surgical therapy consisted of bilateral total adrenalectomy (56 percent of patients). Twelve percent underwent transsphenoidal hypophysectomy and 36 percent had excision of their tumor. No surgical therapy was undertaken in 28 percent. Bilateral total adrenalectomy in patients with a slow-growing malignancy or an unknown tumor secreting ACTH is beneficial in relieving symptoms and prolonging life. Excision of nonmalignant ACTH-producing tumors yields an excellent long-term prognosis.
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PMID:Ectopic ACTH syndrome. Diagnostic and therapeutic aspects. 298 90

240 patients operated on for PHPT in the years 1956-79 were compared with 240 sex and age matched randomized controls to examine the present state of health of the patients. Diseases which occurred in both groups were registered to clarify their possible association with PHPT. Recurring urinary tract infections occurred as often in both groups, though renal stones had naturally been more frequent in the PHPT group. Serum creatinine was elevated in 33 PHPT patients and in 9 controls (p less than 0.001), and renal damage was also more severe in the PHPT patients. Malaise and fatigue occurred in 29 PHPT patients and in 14 controls (p less than 0.05). Medically treated hypertension was found in 95 PHPT patients and in 53 controls (p less than 0.001). Eight PHPT patients and one control had had pancreatitis (p less than 0.01). Peptic ulcer disease and gallstones were registered as frequently in both groups. 16 PHPT patients and three controls had received previous irradiation to the neck region (p less than 0.01). Six PHPT patients, but none of the controls, had had a differentiated non-medullary thyroid carcinoma. Two of the thyroid carcinoma patients had received irradiation. Other malignant diseases were as frequent in both groups. Three controls were hypercalcaemic and had PHPT verified at operation. The prevalence of PHPT in the control group was 1.25%. The state of health was normal in 52% of the PHPT patients and in 68% of the controls (p less than 0.001). The results indicate that PHPT permanently deteriorates the health of the patients and should therefore be diagnosed and treated as early as possible in the course of the disease.
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PMID:The state of health of patients previously operated on for primary hyperparathyroidism compared with randomized controls. 402 76

Multiple Endocrine Neoplasia type 2b (MEN 2b) is a rare syndrome. The principal features are: medullary thyroid carcinoma (MTC), dysmorphism, a ganglioneuromatosis and pheochromocytomas. Eight cases of MEN 2b have been observed at the Institute Gustave Roussy, between 1968 and 1983. Seven involved children under 15 years of age. Eight had a bilateral MTC; six had dysmorphism; six had mucosal tongue neuromas. Six were troubled with visceral ganglioneuromatosis of whom two had intestinal obstruction and one urinary chronic retention. One patient had pheochromocytoma with hypertension. From this experience and other data it appears that: the dysmorphism is frequently poorly interpreted; the visceral ganglioneuromatosis is an early and severe feature; it is important to examine the patient for pheochromocytoma; the MTC must be detected by calcitonin dosage after stimulation, and requires total thyroidectomy; familial screening must be done. To improve the poor prognosis of MEN 2b, early diagnosis and aggressive treatment are necessary.
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PMID:[Multiple endocrine neoplasia type 2b. Clinical, diagnostic and therapeutic aspects]. 614 75


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